tbuckley/cabot-search huggingface dataset:
CPC13 Cutaneous crystal storing histiocytosis
Authors: Geoffrey Brent, Heung Chong, Lorrette Ffolkes, Hari Reddy, Janakan Natkunarajah
Journal: British Journal of Dermatology (2023)

Abstract A 43-year-old woman presented with a 3-year history of a gradually enlarging lesion on the right upper chest. Her comorbidities included ankylosing spondylitis and recurrent anterior uveitis, and she was taking no regular medications. She had no systemic symptoms. She was initially seen by the breast team, who organized an ultrasound that showed chronic inflammation, and a biopsy that showed granulomatous chronic inflammatory tissue. Topical treatment with fucidin was unsuccessful, and she was referred to dermatology. On examination, she had a 3.5 × 4 cm pink subcutaneous nodule on the right upper anterior chest. There was no lymphadenopathy or organomegaly. Investigations revealed low IgA (0.71 g L−1), raised IgM (4.30 g L−1) and mild thrombocytopenia (141 × 109 cells L−1). Full blood count; urea and electrolytes; liver function tests; IgG; lactate dehydrogenase; thyroid stimulating hormone; serum angiotensin-converting enzyme; autoimmune serology, including antinuclear antigen; Borrelia serology; tuberculosis QuantiFERON; ferritin; erythrocyte sedimentation rate; serum protein electrophoresis and urine Bence Jones protein were normal or negative. Chest X-ray and computed tomography of the thorax, abdomen and pelvis did not reveal any evidence of underlying malignancy. Repeat skin biopsy showed a superficial and deep dermal histiocytic infiltrate. At high power, numerous needle-like eosinophilic crystals were seen within the histiocytes. There was a periadnexal and perifollicular infiltrate of lymphocytes and plasma cells. Light chain staining showed that the plasma cells were lambda light chain restricted and were IgM positive and IgG negative. The features were of a crystal-storing histiocytosis. Cutaneous crystal-storing histiocytosis is a rare condition, with only six reported cases. The pathophysiology involves the accumulation of crystalline material within the cytoplasm of histiocytes. It is strongly associated with an underlying haematological malignancy, including multiple myeloma, lymphoplasmacytic lymphoma and monoclonal gammopathy of undetermined significance (Li JJ, Henderson C. Cutaneous crystal storing histiocytosis: a report of two cases. J Cutan Pathol 2015; 42:136–43). In our case, a bone marrow biopsy was not performed given the lack of systemic symptoms, no significant serum paraprotein and normal imaging. The patient is currently being managed with topical clobetasol propionate 0.05% ointment and is under annual haematology surveillance with serum electrophoresis and immunoglobulins.
PA33 Paediatric and adolescent hidradenitis suppurativa: a snapshot of a single centre’s experience
Authors: Laoise Griffin, Katherine Eve, Patsy Lenane, Fiona Browne
Journal: British Journal of Dermatology (2023)

Abstract Hidradenitis suppurativa (HS) is a chronic inflammatory debilitating condition that primarily affects younger people. Its exact prevalence is unknown, but it is estimated to affect at least 1% of the Irish and UK population. Onset is late adolescence and the early 20s in the majority. The impact of early-onset HS (&amp;lt; 18 years) is increasingly recognized, but awareness and knowledge of disease prevalence and severity in Ireland remain sparse. Studies in adults highlight association with a raised body mass index (BMI) and smoking, in addition to an increased prevalence in young females with African American or mixed ancestry. Presently, there is an absence of specific guidelines for management in those aged &amp;lt; 18 years. With rising obesity levels, combined with decreasing pubertal age, we may encounter early-onset HS more often in future. We present a snapshot of patients with HS attending a single paediatric hospital who were considered for systemic therapy. Ten patients were included (eight females). Mean (SD) age of symptom onset was 12.4 (1.9) years, mean (SD) age of presentation was 14 (2.1) and mean duration of symptoms prior to presentation was 1.5 years. Nine received systemic therapy (nine antibiotics and four biologics). Disease severity, defined by Hurley stage, was mild in one, moderate in five and severe in four. All patients had achieved puberty, one was defined as early-onset (age 10 years) and another as precocious (age 9 years). The BMIs varied: one was normal, two were above a healthy weight, five were very overweight and three were severely overweight according to UK centiles. Most patients experienced comorbidities, including, but not limited to, Crohn disease (n = 1), hepatic steatosis (n = 2), pilonidal sinus (n = 3) and acne (n = 5). One had diabetes, two impaired fasting glucose and one hypercholesterolaemia. No patient reported smoking. Passive smoking and blood pressure were not documented. Four patients had positive family histories. Six patients identified as Black Irish (one Irish Caribbean and five Irish Nigerian), two were White Irish, one patient was from India and one was from Poland. We describe a group of individuals aged ≤ 16 years, the majority of whom had moderate–severe HS. They had similar comorbidities to internationally reported literature in early-onset HS. More skin of colour ethnicities are represented than seen in the general Irish population, which probably reflects both the catchment area and severity of the skin conditions experienced. The limitations of our study include under-representation of milder disease due to an inability to perform a full cross-sectional study at present. Further prospective studies are required to capture the prevalence and characteristics of HS fully in our population of children and young adults. Considering the lifetime impact of this condition, guidelines for the management of HS under the age of 18 years should be considered.
BI30 Remission of a large plantar verruca vulgaris in an immunocompromised patient postincisional punch biopsy
Authors: Paula Beatty, Marta Costa Blasco, Lisa Murphy, Anna Wolinska, Orla McFeely, Claire Doyle, Madonna Andrawis, Anne-Marie Tobin, Caitriona Hackett
Journal: British Journal of Dermatology (2023)

Abstract We report the case of a 41-year-old renal transplant recipient who has been attending dermatology for skin surveillance following a cadaveric renal transplant. His immunosuppressive treatment consists of tacrolimus, mycophenolate mofetil and prednisolone 5 mg once daily. His primary dermatological complaint was that of a large wart on the plantar aspect of his right foot. Given the location of this lesion, there was significant associated pain, which also resulted in a functional impediment, impacting on his ability to exercise or work. On examination, he had a large, hyperkeratotic, papillomatous nodule measuring 3.5 × 3 cm on the plantar aspect of his right foot, clinically in keeping with verruca vulgaris. This had been recalcitrant to extensive treatment, including topical salicylic acid, 5-fluorouracil, cantharidin and numerous courses of cryotherapy and paring over the previous 6 years. Following administration of xylocaine 1% with adrenaline as the local anaesthetic, we performed three incisional 4-mm punch biopsies through the centre of the wart to the depth of the dermis. We ensured visualization of haemorrhage at the base of each punch incision. The biopsy cores were left in situ and the wound was dressed with petroleum jelly, Kaltostat® and Opsite. At his 6-week follow-up review, the verruca vulgaris had completely resolved. No postprocedural complications were noted by the patient. Further skin surveillance was scheduled for 6 months later, at which time no recurrence of this verruca vulgaris had occurred. Verruca vulgaris is a benign papillomatous proliferation of epidermal cells driven by human papillomavirus (HPV) infection, commonly acquired in childhood. For many patients, verruca vulgaris will spontaneously regress. However, it can represent a therapeutic challenge when persistent, as it is recalcitrant to multiple treatment modalities, particularly in immunosuppressed patients. To our knowledge, our case is the first documented resolution of a giant plantar wart in an immunocompromised patient using such an approach. Renal transplant patients represent a unique cohort in relation to verruca vulgaris, whereby impaired cell-mediated immunity can increase their susceptibility to HPV, thereby reducing effective treatment options and meaning definitive clearance may never be achieved. Our case highlights that incisional punch biopsy may represent an accessible and cost-effective approach to the treatment of verruca vulgaris in immunosuppressed patients, where alternative treatment options would necessitate numerous hospital visits.
CPC03 Triad of generalized rash, peripheral eosinophilia and coexisting obstructive airway disease
Authors: Alex Willsher, Livia Soriano, Salma Selim, Wei Chen Fong, Adam Fityan
Journal: British Journal of Dermatology (2023)

Abstract An 87-year-old woman was referred to the acute dermatology service with a 1-week history of a generalized painful rash. She was under the care of the medical team for treatment of pneumonia with symptoms of a cough, fevers and shortness of breath. Her past medical history included adult-onset asthma, hyponatraemia and hypertension. She had never smoked. On examination, she had erythematous smooth papules and plaques on her limbs and torso with palmoplantar involvement, alongside purple macules and patches where older lesions were present. There was no lymphadenopathy and no mucosal involvement. She had an oxygen requirement of 2 L per minute via nasal cannula. Initial blood tests showed eosinophils of 2.8 × 109 cells L−1 and a C-reactive protein (CRP) of 77 mg L−1. A chest radiograph showed bilateral ill-defined shadowing. Two skin punch biopsies were performed for histology and immunofluorescence. Vasculitis screen identified positive antinuclear antibodies (anti-Ro) and reduced complement C4 with negative antineutrophil cytoplasmic antibody (ANCA). The patient remained unwell with increasing CRP and peripheral eosinophilia (up to 9.2 × 109 cells L−1) with rash progressing to exhibit a reticular pattern. Alongside bilateral patchy ground glass opacification on chest computed tomography, a clinical diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) was made. Differential diagnoses included viral exanthem, erythema multiforme and urticarial vasculitis. Screening for cardiac, renal and sinus involvement was undertaken. The patient improved clinically following the initiation of systemic steroids and the peripheral eosinophilia returned to baseline. Skin histology reinforced the clinical diagnosis of EGPA, demonstrating a dermal and perivascular eosinophilic infiltrate with focal extension into vessel walls. EGPA is a rare small- to medium-sized vessel vasculitis with an annual incidence of 0.9–2.4 per million and a median age at diagnosis of 49–59 years. The clinicopathological hallmarks, as in this case, are asthma with peripheral eosinophilia and pulmonary eosinophilic infiltrates. Median time from onset of asthma to presentation with EGPA is 5–9 years. EGPA remains a clinical diagnosis, reinforced by serological and histological findings, although ANCA is reported to be negative in 60–70% of cases. Systemic steroids are the first-line therapy, with a role for second-line immunosuppressants such as methotrexate and rituximab. The key learning points from this case are to keep an open mind in considering a systemic cause for cutaneous manifestations, especially when the patient does not fulfil the usual demographic picture.
BT23 Outcomes of skin lesion referrals assessed via teledermoscopy
Authors: Iman Hamid, Isabelle Vandormael, Caroline Mills, Ausama Atwan
Journal: British Journal of Dermatology (2023)

Abstract Skin lesion referrals from primary care comprise a significant proportion of the dermatology workload. Urgent suspected cancer (USC) referrals are prioritized, which adds strain to outpatient capacity and impacts on waiting lists for less urgent and routine referrals. Our department provides a teledermoscopy diagnostic service to allow for timely diagnosis of skin lesions. This results in fast detection of USC and a reduction in unnecessary hospital visits for patients with benign lesions. The aim of this study was to review the outcomes of USC and routine referrals received from primary care and assessed via our teledermoscopy service. We randomly selected 381 skin lesion referrals triaged for teledermoscopy between January and March 2022. In total, 189 (49.6%) of these were referred as routine and 192 (50.4%) were urgent/USC. High-resolution clinical and dermoscopic photographs of all skin lesions were taken by qualified clinical photographers and stored in the patients’ electronic records. Two separate consultant dermatologists reviewed the images along with the information provided in the referrals for diagnosis. A total of 47 diagnoses were observed in both cohorts. Only 16% of patients referred as urgent/USC had suspected high-risk cancer, including squamous cell carcinoma (SCC; 8.8%), melanoma (5.7%) and lentigo maligna (1.5%). The remainder of urgent/USC referrals were identified as nonurgent or benign, with an overwhelmingly low risk of basal cell carcinoma (23.9%), seborrhoeic keratoses (20.8%) and benign naevi (9.3%). For routine referrals, 11 patients (5.8%) were suspected to have USC. Of these, five had histological confirmation of melanoma (n = 3), SCC (n = 1) and melanoma in situ (n = 1). Of all referrals, 44.6% were reassured and discharged; 20.8% required clinic review for a diagnostic biopsy or treatment; 20.7% were booked directly for minor surgery; 8.3% were referred to the community local enhanced services for the treatment of low-risk lesions on the torso and limbs; 1.3% were referred to other specialties; and 4.1% required digital monitoring by repeating photographs 3–6 months later. An ever-increasing prevalence of skin cancer, together with suboptimal dermatology training for primary care healthcare professionals, anxiety over misdiagnosing malignancy and pressure from anxious patients all contribute to the pressure on dermatology services. We have shown that a teledermoscopy service is a useful tool for triaging skin cancer referrals to maximize the utilization of outpatient capacity and ensure timely treatment for patients with USCs.
PD02 Home daylight photodynamic therapy
Authors: Marese O’Reilly, Carol Goodman, Susan Yule, Andrea Lesar, Ewan Eadie, Sally Ibbotson
Journal: British Journal of Dermatology (2023)

Abstract Actinic keratoses (AK) on the face and scalp are often extensive and are a major cause of morbidity and associated with increased risk of skin cancer. Field-directed treatment approaches, such as daylight photodynamic therapy (DPDT) are required, and this has been shown to be effective, well-tolerated and feasible in the UK between April and September. We have used DPDT since 2013, with good outcomes. Patient engagement indicated that convenience and tolerability were rated as highly as efficacy, and we therefore developed an entirely home-based approach ‘home DPDT (H-DPDT)’ in 2021. Working with an art-and-design student, we developed a fully recyclable home kit to enable patients to self-treat with H-DPDT entirely in their home environment. Patients were selected by a dermatologist and then received nurse-led training prior to treatment. In-person review after the second treatment was undertaken to assess response and decide if further treatment was needed, and telephone follow-up was undertaken after the first and third treatments. Final clinician review was undertaken 3–6 months after the last treatment. We reported on implementation in 2021 and introduced this into our routine PDT service options, and we now wish to report our findings from 2021–22. Seventeen patients (82% male) were treated in 2021–2022 for AK on the head and neck. Each patient completed an average of four (range 2–5) treatments. Clinical response was good (≥ 75% response) in nine of 15 (60%) patients assessed. Response was moderate in one (7%) patient (50–75% response) and the remaining five (33%) had a poor response (&amp;lt; 50% response). Treatment success correlated with grade of disease. Patients with more hyperkeratotic disease had poorer outcomes. In 2022, five patients responded to the routine daylight PDT questionnaire. Of these, two rated the H-DPDT as Excellent and the other three rated it as Good. Four patients stated that they had tried other AK treatments, two of which thought that H-PDT was better. One patient felt alternative treatments were more effective but they tolerated H-PDT better. Three of the five patients expressed a preference for H-DPDT for repeat treatment and would recommend it. This innovative H-DPDT service enables patients to self-treat in their home environment, and the initial results showed high rates of patient satisfaction. Treatment outcomes were better for those with milder disease, highlighting the need for careful patient selection and good surface preparation. As a proof of concept, H-DPDT is a feasible therapeutic option and empowers patients, but it may have more of a place in the treatment of milder AK disease, and further work to optimize this approach is indicated.
P10 Blastic plasmacytoid dendritic cell neoplasm: two representative cases
Authors: Neha Taneja
Journal: British Journal of Dermatology (2023)

Abstract Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a malignant proliferation of plasmacytoid dendritic cells, representing &amp;lt; 1% of primary cutaneous lymphomas. It most commonly presents in the skin and has a poor prognosis with median survival of 1 year. The fifth World Health Organization classification of haematolymphoid tumours encompasses two entities in plasmacytoid dendritic cell neoplasms: BPDCN and ‘mature plasmacytoid dendritic cell neoplasm associated with myeloid neoplasm’. We present two cases, one representing each subset. Our first case was a 58-year-old man who presented with an asymptomatic, rapidly progressive, skin coloured-to-ery­thematous and hyperpigmented firm papules, nodules and plaques over the trunk, upper limbs and face of 2.5 months’ duration. This was associated with loss of appetite, loss of weight and leucopenia (2180 cells μL−1). Skin biopsy revealed a grenz zone with pandermal infiltration by large cells with high nuclear : cytoplasmic ratio, vesicular nuclei and prominent nucleoli, and frequent mitotic figures. Immunohistochemistry (IHC) was leucocyte common antigen (LCA)+ but CD3–, CD20–, myeloperoxidase (MPO)– and CD117–. Bone marrow biopsy showed a similar infiltrate of cells, but the peripheral blood smear and flow cytometry was within normal limits. Further IHC was CD4+, CD56+, CD43+ and CD123+, establishing a diagnosis of BPDCN, and the patient was initiated on the Hoelzer protocol with 80% clearance of lesions after two cycles of chemotherapy. The second case was a 60-year-old woman who presented with asymptomatic firm skin-coloured nodules on her scalp, trunk, extremities and oral mucosa, associated with a low-grade fever, fatigability, weight loss, generalized lymphadenopathy and hepatosplenomegaly of 4 months’ duration. Histopathology showed similar features to the first case, with IHC being negative for CD3, CD20, CD138 and MPO. However, cells were LCA+, CD4+, CD56+ and CD43+. Bone marrow showed 77% acute leukaemia blasts with a MPO+ result, establishing diagnosis of acute myeloid leukaemia, suggestive of a mature plasmacytoid dendritic cell neoplasm associated with myeloid neoplasm. To conclude, BPDCN is an aggressive neoplasm that commonly presents in the skin and should be suspected by dermatologists when screening IHC for lymphoid and myeloid cell lines is negative in a suspected case of leukaemia cutis. A panel of CD4/CD56/CD43/CD123 may be used to establish the diagnosis.
007 Vitiligo is associated with a reduction in incidence of melanoma and nonmelanoma skin cancer: UK population-based cohort study
Authors: John Ferguson, Viktoria Eleftheriadou, John Nesnas
Journal: British Journal of Dermatology (2023)

Abstract Vitiligo is a common, acquired depigmenting skin disease. There have been concerns that vitiligo and its treatment may increase the risks of skin cancer. In a large UK population-based study, we aimed to establish whether vitiligo is associated with an increased risk of skin cancer. We used routinely collected electronic medical records of adults contributing to the Optimum Patient Care Research Database (2010–2020). Cases of vitiligo were identified using specific diagnosis codes. Those with other depigmenting conditions were excluded. Vitiligo cases were age and sex matched with up to four controls registered at the same general practice and with at least one primary care interaction in the preceding year. The primary outcome was risk of skin cancer; comprisied of melanoma, squamous cell carcinoma (SCC) and basal cell carcinoma (BCC). Individual skin cancers and actinic keratoses (AK) were included as secondary outcomes. Associations between vitiligo and skin cancer were estimated using Cox regression, with adjustment for age, sex, socioeconomic deprivation, ethnicity, body mass index, smoking and alcohol status, immunotherapy, phototherapy and 13 common comorbidities. Prespecified subgroup analysis comprised stratification by sex, age group (18–45 years, 45–65 years and &amp;gt; 65 years), ethnicity (White and non-White) and socioeconomic deprivation. In total, 14 976 patients with vitiligo were matched to 54 392 controls. Over the 10-year study period, 133 patients with vitiligo and 783 controls developed new-onset skin cancer. Vitiligo was associated with a 38% reduced risk of any new-onset skin cancer [adjusted hazard ratio (aHR) 0.62, 95% confidence interval (CI) 0.51–0.74; P &amp;lt; 0.001]. This risk reduction was observed for all individual skin cancer types: melanoma (aHR 0.38, 95% CI 0.23–0.64; P &amp;lt; 0.001), SCC (aHR 0.65, 95% CI 0.48–0.87; P = 0.004), BCC (aHR 0.65, 95% CI 0.51–0.83; P &amp;lt; 0.001) and AK (aHR 0.86, 95% CI 0.75–0.98; P = 0.028). In a subgroup analysis, vitiligo was associated with a reduction in skin cancer risk in people identifying as White (aHR 0.68, 95% CI 0.55–0.84; P &amp;lt; 0.001), but there was no association in those identifying as non-White (aHR 0.93, 95% CI 0.44–1.98). Vitiligo-associated reductions in skin cancer risk were consistently observed in the other prespecified subgroups. Vitiligo is associated with a strong protective effect against common types of skin cancer. Vitiligo-associated risk reduction is most marked for melanoma (62% lower risk). Protective effects associated with vitiligo may be restricted to people with light skin, although skin cancers in the those with darker skin were uncommon, which requires further study. FundingThis research was sponsored by Pfizer. Medical writing and statistical support was provided by Momentum Data and was funded by Pfizer.